case 1 srdp, sibiu, october 20121. authors: sabina zurac 1, razvan andrei 1, tiberiu tebeica 1,...

Case 1Case 1

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Authors:Authors:

Sabina ZuracSabina Zurac 1 1, Razvan Andrei, Razvan Andrei11, Tiberiu Tebeica, Tiberiu Tebeica11, , Florica StaniceanuFlorica Staniceanu11, ,

Adrian Rebosapca, Bogdan AndreescuAdrian Rebosapca, Bogdan Andreescu22

11PathologyPathology22Plastic Surgery Plastic Surgery

Colentina University Hospital, Bucharest, RomaniaColentina University Hospital, Bucharest, Romania

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MaleMale

82 yrs old82 yrs old

Ulcerated tumor, left temporal area, max Ulcerated tumor, left temporal area, max diameter 1.5 cmdiameter 1.5 cm

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vimentin

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IHC – negative markersIHC – negative markers

AE1-AE3AE1-AE3MNF116MNF116CK7CK7CK14CK14CK8/18CK8/18CK5/6CK5/63434ββE12E12BER-EP 4BER-EP 4P63P63HMB45HMB45Melan AMelan AT311T311S100S100

SMASMADesminDesminHHF35HHF35CLACLACD20CD20CD3CD3CD30CD30EMAEMACD34CD34CD31CD31CD105CD105F VIIIF VIII

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Atypical fibroxantoma

Atypical fibroxantoma

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Atypical fibroxantomaAtypical fibroxantoma

highly pleomorphic tumor histologyhighly pleomorphic tumor histology

Benign clinical behaviourBenign clinical behaviour– ““little, little, if anyif any, potential for metastasis”, potential for metastasis”– ““benign tumor when benign tumor when strictstrict diagnostic criteria diagnostic criteria

are applied”are applied”Barnhill Dermatopathology, McGraw Hill, 2010Barnhill Dermatopathology, McGraw Hill, 2010

diagnosis of exclusiondiagnosis of exclusion

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Atypical fibroxantomaAtypical fibroxantoma

Clinical appearance – not characteristicClinical appearance – not characteristicold adults old adults sun-damaged skin sun-damaged skin Associated pathologyAssociated pathology– xeroderma pigmentosum xeroderma pigmentosum – ImmunosuppressionImmunosuppression

Rapid growthRapid growthRather small (< 1.5 cm)Rather small (< 1.5 cm)

!!! Rarely identified solely on macroscopic aspect (MM, BCC, !!! Rarely identified solely on macroscopic aspect (MM, BCC, SCC)SCC)

!!!Young pts, no actinic damage – more likely atypical !!!Young pts, no actinic damage – more likely atypical

dermatofibromadermatofibromaSRDP, Sibiu, october 2012SRDP, Sibiu, october 2012 1313

Atypical fibroxantomaAtypical fibroxantoma

Ultraviolet-induced p53 mutationsUltraviolet-induced p53 mutations

AFX vs undifferentiated high grade AFX vs undifferentiated high grade sarcomasarcoma– CGH analysisCGH analysis

Common alterations: deletions on chromosomes Common alterations: deletions on chromosomes 9p and 13q. 9p and 13q.

Differences: deletions on 1q, 3p, 5q, 11p, 11q, Differences: deletions on 1q, 3p, 5q, 11p, 11q, gains on 7q, 12q; high level gains on 5p and 11q.gains on 7q, 12q; high level gains on 5p and 11q.Mihic-Probst et al Anticancer Res. 2004 Jan-Feb;24(1):19-26.Mihic-Probst et al Anticancer Res. 2004 Jan-Feb;24(1):19-26.

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Atypical fibroxantomaAtypical fibroxantoma

MicroscopyMicroscopysymmetric, well-circumscribedsymmetric, well-circumscribedexophytic exophytic Dermal, very superficial adipose extensionDermal, very superficial adipose extensiongrenz zonegrenz zoneulceration.ulceration.NO pagetoid spreadNO pagetoid spreadepidermal collaretteepidermal collaretterelatively storiform or fascicular patternrelatively storiform or fascicular pattern

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Atypical fibroxantomaAtypical fibroxantoma

MicroscopyMicroscopyTumor cells Tumor cells – highly pleomorphic (marked irregular and hyperchromatic highly pleomorphic (marked irregular and hyperchromatic

nuclei, bizarre multinucleated tumor giant cells)nuclei, bizarre multinucleated tumor giant cells)– uniform spindle cellsuniform spindle cells

uniform cytologic atypia in tumor mass.uniform cytologic atypia in tumor mass.Mitoses: frequent, numerous, atypical.Mitoses: frequent, numerous, atypical. multinucleated giant histiocytes, some Touton-typemultinucleated giant histiocytes, some Touton-typeNO extensive extension in adipose tissueNO extensive extension in adipose tissueNO necrosis and vascular or perineural invasionNO necrosis and vascular or perineural invasion

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Atypical fibroxantomaAtypical fibroxantoma

Immunophenotype highly nonspecificImmunophenotype highly nonspecific– + vimentin, + vimentin, αα1-AT, 1-AT, αα1-ACT, CD68, SMA, 1-ACT, CD68, SMA, – +CD10, +CD117+CD10, +CD117– Large panels to exclude dif malignancies.Large panels to exclude dif malignancies.– Aware to reactive/entrapped cells: epithelial, Aware to reactive/entrapped cells: epithelial,

Langherhans, vessels Langherhans, vessels Longacre TA et al. Longacre TA et al. Am J Surg Pathol. Am J Surg Pathol. 1993;17:1199-1209.1993;17:1199-1209.

Singh M et al. J Oral Maxillofac Surg. 2012 Apr 9.Singh M et al. J Oral Maxillofac Surg. 2012 Apr 9.

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Atypical fibroxantomaAtypical fibroxantoma

““If strict criteria are followed, atypical fibroxanthoma may be If strict criteria are followed, atypical fibroxanthoma may be regarded as benign neoplasms cured by simple excision”regarded as benign neoplasms cured by simple excision”

Barnhill Dermatopathology, McGraw Hill, 2010Barnhill Dermatopathology, McGraw Hill, 2010Local recurrences, no metastases Local recurrences, no metastases

– series of 140 atypical fibroxanthomasseries of 140 atypical fibroxanthomasFretzin, Helwig. Fretzin, Helwig. Cancer.Cancer.1973;31:1541-1552.1973;31:1541-1552.

– series of 171 atypical fibroxanthomasseries of 171 atypical fibroxanthomasBeer TW et al Am J Dermatopathol. 2010 ;32:533-40Beer TW et al Am J Dermatopathol. 2010 ;32:533-40

IHC tests to exclude malignant fibrous histiocytoma, angiosarcoma, IHC tests to exclude malignant fibrous histiocytoma, angiosarcoma, malignant melanoma, and squamous cell carcinoma malignant melanoma, and squamous cell carcinoma Morphologic alterationsMorphologic alterations– NO extensive extension in adipose tissueNO extensive extension in adipose tissue– NO necrosis and vascular or perineural invasionNO necrosis and vascular or perineural invasion

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